TWO AUTOPSY CASES OF GLYCOGEN STORAGE DISEASE ‐CIRRHOTIC TYPE
- 1 February 1973
- journal article
- Published by Wiley in Acta Pathologica Japonica
- Vol. 23 (1) , 211-223
- https://doi.org/10.1111/j.1440-1827.1973.tb00786.x
Abstract
The present report deals with the results of our study on two autopsy cases in whom hepatosplenomegaly and disturbances of the liver function had been noticed in early life. Both died at the age of 23 months and 30 months old, respectively. The necropsy of the cases had demonstrated the depositions of abnormal glycogen (polysaccharide) In the liver, heart and the reticuloendothelial system, and cirrhosis of the liver. Histoche‐mically, the stored material resembled glycogen but it showed some unusual properties, particularly its slow digestion by amylase as well as the tone of a mauve color with Iodine and the affinity for ruthenium red. These findings suggested that these two cases resemble very closely glycogen storage disease of type IV (Andersen's disease), which is a very rare disease.This publication has 7 references indexed in Scilit:
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